Familiar non-chromaffinic paragangliomas (glomus tumors). Clinical and genetic aspects (abridged)

Abstract

The presence of glomus tumors in a large family was investigated by clinical and angioscintigraphic screening methods. Of 295 members of this family 162 persons participated in the study. A total of 47 tumors of the head and neck region were found in 26 patients. The study revealed a preponderance for the male sex, a fairly equal distribution of the different locations in the head and neck and a marked tendency towards multiplicity. The inheritance is autosomal dominant, with a clear increase of the penetrance with age.